Surgical Intervention in Extensive Splanchnic Artriovenous Thrombosis Associated with JAK-2 Mutation
Faez Ayoob, M.D, Zachary Chadnick, MD, Charles Fasanya, M.D, Kuldeep Singh, M.D.
Staten Island University Hospital, Staten Island, NY, USA.
Introduction: Essential thrombocytosis (ET) is a rare condition caused by an overproduction of platelets. Overwhelming number of patients are asymptomatic and are managed conservatively. We present three cases of complications related to ET that required surgical management and review of the literature pertaining to ET.Methods: On September 26, 2013 a literature search was performed on PubMed and Google scholar. The search terms used were “essential thrombocytosis”, “JAK-2 mutation” (thrombosis or infarction or embolus) and (splenic or mesenteric)”. Only articles in English were included; 15 articles and 17 case reports were found. All the articles were obtained through the library and were reviewed by all the authors. A repeat search was performed on September 1, 2018 using the same criteria with an additional case report found and new diagnostic criteria being put in place for the diagnosis.Results: First case was a 75-year-old female with a hypercoagulable state presented with abdominal pain and was noted to have acute splenic and mesenteric infarcts she underwent splenectomy followed by partial enterectomy as definitive management. Second patient was a 68-year-old female presented with distal mesenteric small vessel thrombosis and bowel ischemia underwent an exploratory laparotomy followed by interval small bowel resection and primary anastomosis. Third case was a 65-year-old female presented with free air, portal vein thrombosis and proctosigmoid ischemia; underwent proctosigmoidectomy and end colostomy. All cases received therapeutic anticoagulation and oral chemotherapy for their essential thrombocytosis. All patients were noted to have JAK-2 mutation,other embolic causes were ruled out.Conclusion: ET has a rare association with large vessel thrombosis in both the arterial and venous systems that may require surgical intervention, especially in the elderly population. JAK-2 mutations should be considered as an etiological risk factor.. 
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